Detailed characteristics of included studies describing spirometric indices of primary ciliary dyskinesia patients, stratified by age group of patients
| First author [ref.] | Country | Year of publication | Patients n | Age years | Type of study | Study design | Diagnostics | Symptoms stratified by age | Longitudinal data | FEV1 | FVC |
| Children | |||||||||||
| Al Saadi [51] | Saudi Arabia | 2013 | 22 | 10.86±3.09# | Prospective | Case–control | EM+HFVM | NA | − | + | − |
| Davis [55] | USA/Canada | 2015 | 118 | 8 (5–11)¶ | Prospective | Case series | EM or genetic analysis | NA | − | + | − |
| Gokdemir [58] | Turkey | 2014 | 24 | 12.9±2.7# | Prospective | Clinical trial | EM or Kartagener | NA | − | + | + |
| Karadag [61] | UK | 1999 | 21 | 10.8±3.2# | Retrospective | Case–control | EM, HFVM | NA | − | + | + |
| Koh [62] | Korea | 2000 | 19 | 12 (7–16)+ | Prospective | Clinical trial | EM | NA | − | + | − |
| Maglione [63] | Denmark, Italy and UK | 2014 | 158 | 8.7 (4.2–17.4)§ | Retrospective | Cohort | According to consensus | NA | + | + | + |
| Paff [68] | The Netherlands | 2013 | 25 | 10.7 (7.1–14.5)¶ | Prospective | Case–control | According to consensus | NA | − | + | − |
| Adults | |||||||||||
| Frija-Masson [57] | France | 2017 | 78 | 34.8 (28.6–47.1)¶ | Retrospective | Case series | EM, genetic analysis or Kartagener | NA | + | + | + |
| Horvath [60] | UK | 2003 | 14 | 35±4.6# | Prospective | Case–control | EM, HFVM | NA | − | + | + |
| Mossberg [65] | Sweden | 1983 | 24 | 30 (19–47)§ | Prospective | Case series | Clinical suspicion | NA | − | + | − |
| Both children and adults | |||||||||||
| Amirav [52] | Israel | 2016 | 13 | 10 (5–43)¶ | Retrospective | Case series | All tests, including genetic analysis | − | − | + | − |
| Boon [53] | Belgium | 2014 | 122 | 17.7 (9.5–28.1)¶ | Retrospective | Case series | EM, HFVM, genetic analysis | − | − | + | + |
| Cohen-Cymberknoh [54] | Israel | 2014 | 14 | 15.9±8.6# | Retrospective | Case series | EM, HFVM, nNO, genetic analysis | − | − | + | − |
| Ellerman [56] | Denmark | 1997 | 24 | 21±13.6# | Prospective | Case series | HFVM | + | + | + | + |
| Hellinckx [59] | Belgium | 1998 | 11 | 15.2±7# | Prospective | Case series | EM, HFVM | + | + | + | − |
| Mahut [64] | France | 2006 | 16 | 13.5 (12–17)¶ | Retrospective | Case series | Clinical, EM | + | − | + | − |
| Noone [4] | USA and Canada | 2004 | 78 | Adults: 36 (19–73)+; children: 8 (1–17)+ | Prospective | Case series | EM, HFVM, genetic analysis | + | − | + | − |
| Nyilas [66] | Germany and Switzerland | 2017 | 49 | 14.7±6.6# | Prospective | Case–control | All tests | − | − | + | + |
| Olm [67] | Brazil | 2011 | 10 | 13.4±4.7# | Prospective | Case–control | EM+HFVM | + | − | + | + |
| Pifferi [69] | Italy | 2015 | 45 | 14 (22.25)¶ | Prospective | Case–control | EM, HFVM, nNO | − | − | + | + |
| Shah [70] | UK | 2016 | 118 | 23.5 (10–36)¶ | Retrospective | Case series | EM, HFVM, nNO, 2% clinical | − | + | + | − |
| Tolusakow [71] | Germany | 1981 | 21 | 3–43ƒ | Retrospective | Case series | Clinical suspicion | − | − | + | + |
| Vallet [72] | France | 2013 | 41 | 6 (3–11)¶ | Retrospective | Case series | EM, HFVM | − | + | + | + |
| Yiallouros [73] | Cyprus | 2015 | 20 | 0.1–58.4ƒ | Retrospective | Case series | EM or HFVM+nNO | + | + | + | + |
FEV1: forced expiratory volume in 1 s; FVC: forced vital capacity; EM: electron microscopy; HFVM: high-frequency video-microscopy; NA: not applicable; nNO: nasal nitric oxide. Age is reported in different formats depending on the available information: #: mean±sd; ¶: median (interquartile range); +: median (range); §: mean (range); ƒ: range.